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Clinical skills Yr2 > Haematological emergencies > Flashcards

Haematological emergencies Flashcards

1
Q

Does neutropenic sepsis always present with febrile neutropenia?

A

No

Patient can have neutropenia without fever

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2
Q

What are the 2 defining features of neutropenic sepsis?

A

Absolute neutrophil count is 0.5 x 10(9)/L or lower

Temperature greater than 38 degrees Celsius OR any symptoms/signs of sepsis

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3
Q

What is the main reason why neutropenic sepsis is a life-threatening emergency?

A

Neutropenic patients already have weakened immune system and are more likely to contract bacterial infections which can become disseminated and fatal

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4
Q

Give 4 haematological risk factors for neutropenic sepsis?

A

Recent chemotherapy (usually within 14 days): Biggest risk factor

Immunotherapy with cytotoxic drugs

Stem cell transplant patient

Bone marrow disorders

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5
Q

What treatment pathway is used for neutropenic sepsis?

A

Sepsis 6 care bundle

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6
Q

How does a patient with neutropenic sepsis start the sepsis 6 care bundle treatment?

A

Call 999 ambulance for emergency transfer to hospital, ideally within 1 hour of neutropenic sepsis recognition

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7
Q

What is included in the sepsis 6 care bundle, to treat neutropenic sepsis?

A

3 diagnostic and 3 therapeutic steps delivered within 1 hour after initial recognition of sepsis

  1. Oxygen administration
  2. Two sets of blood cultures
  3. Venous blood lactate
  4. Fluid resuscitation
  5. Appropriate antibiotics
  6. Monitor observations
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8
Q

How should oxygen be administered to treat neutropenic sepsis?

A

Give oxygen therapy to people with reduced oxygen saturation (below 92%) or with an increase in oxygen requirement over baseline, to maintain oxygen saturation above 94% unless contraindicated

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9
Q

How are the two blood cultures taken from the patient, in neutropenic sepsis treatment?

A

Paired cultures from a peripheral/central line in situ and periphery

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10
Q

What is the first line antibiotic for neutropenic sepsis and why?

A

Continuous IV piperacillin with tazobactam (tazocin)

Provides anti-pseudomonal cover

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11
Q

What hormone drug can you administer to patients with neutropenic sepsis and why?

A

Recombinant G-CSF eg. Filgastrim

Can be used as treatment and prophylaxis to prevent neutropenia

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12
Q

If a patient with neutropenic sepsis is allergic to penicillin, which antibiotic should be administered instead of tazocin?

A

Second-line: IV Meropenem

Tazocin contains penicillin so cannot be used

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13
Q

What are the 4 emergency sickle cell crises?

A

Vaso-occlusive crisis

Aplastic crisis

Sequestration

Acute chest syndrome

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14
Q

What is a vaso-occlusive crisis in SCD patients?

A

Sickled RBCs get stuck in microcirculation eg. capillaries and cause ischemic injury to organs and tissue

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15
Q

What is an aplastic crisis in SCD patients?

A

Bone marrow suppression usually due to infection of parvovirus B19 which stops RBC production for a short time

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16
Q

What is acute splenic sequestration crisis in SCD patients?

A

Sickled RBCs block blood vessels exiting spleen, so spleen becomes engorged with blood

Causes severe anaemia and life-threatening circulatory collapse

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17
Q

What is acute chest syndrome in SCD patients?

A

Vaso-occlusive crisis in pulmonary vasculature

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18
Q

What is the main symptom of sickle cell crises?

A

Sudden and intense pain throughout whole body

19
Q

What is the management for sickle cell crisis (HOP to it)?

A

H: Hemodilution (IVF, PRBCs)

O: Oxygen supplementation

P: Pain relief

20
Q

In sickle cell crisis, how is hemodilution managed in 2 steps?

A

Exchange transfusion: Machine replaces sickled blood with donor PRBCs

Intravenous fluids: Slows sickling process

21
Q

In sickle cell crisis, how is oxygen supplementation managed?

A

Provide oxygen if below 95% on room air

22
Q

In sickle cell crisis, how is pain relief managed?

A

Mild pain: Paracetamol/ibuprofen/naproxen

Moderate pain: Codeine, tramadol

Severe pain: Morphine, oxycodone

23
Q

What are the 3 defining signs of acute chest syndrome in SCD patients?

A

Fever

Respiratory symptoms

Pulmonary infiltrate (opacity) on chest x-ray

24
Q

What is TTP and why is it a haematological emergency?

A

Clots form in microcirculation which blocks blood flow to heart, kidney, brain and other organs

25
Q

What is the classic pentad of TTP symptoms? (FAT RN)

A

Fever
Anaemic symptoms due to MAHA
Thrombocytopenia: Purpura or bleeding
Renal failure: Thirst, frequent urination
Neurological symptoms

26
Q

Which 5 investigations should be immediately done to confirm TTP?

A

FBC: Marked thrombocytopenia, haemolytic
anaemia markers eg. reticulocytosis, low LDH, low haptoglobins

Coagulation screen: Normal

Peripheral blood film: Schistocytes, polychromasia, thrombocytopenia

ADAMTS13 level: Very low

Urinalysis and renal function tests

27
Q

What is the mainstay emergency treatment of TTP, whether it is congenital or immune-mediated?

A

Plasma exchange: Donor plasma with normal ADAMS13 levels replaces patient’s plasma which contains autoantibodies and no ADAMS13

28
Q

Which 3 medications can either be administered to treat TTP?

A

Steroids eg. methylprednisolone: Reduces antibody level and immune response to ADAMS13

Rituximab: Stops ADAMS13 from being broken down

Caplacizumab: Stops platelets from sticking to von Willebrand factor

29
Q

Which supportive medicines are administered to treat TTP?

A

Folic acid

Omeprazole

Dalteparin (heparin injection)

30
Q

What is Tumour Lysis Syndrome (TLS)?

A

Tumour cell breakdown (usually caused by cancer treatment) release contents into bloodstream

31
Q

Give 4 common risk factors for TLS?

A

High-grade cancers eg. Burkitt’s lymphoma, acute leukaemias

Recent chemotherapy

Renal issues

Dehydration

Conditions causing hyperuricemia

32
Q

What is the classic tetrad of lab findings, and one additional finding for TLS? (PLUCK)

A

P: Hyperphosphatasemia

L: LDH low

U: Hyperuricemia

C: Hypocalcemia

K: Hyperkalemia

33
Q

What are some symptoms of TLS?

A

Urinary symptoms eg. haematuria, dysuria

Hypocalcemia: Cramps, vomiting, altered mental status, spasms

Hyperkalemia: Weakness, paralysis

Arrythmias, sudden death

34
Q

How do you manage TLS?

A

Hyperuricemia: Rasburicase, if condraindicated give allopurinol

Hyperphosphatemia: IV fluids and monitor high urine output, if uncontrolled use dialysis

Hypocalcemia: Cardiac monitoring unless symptomatic, then give calcium gluconate

Hyperkalemia: Cardiac monitoring unless severe, then haemodialysis

35
Q

What is Acute Promyelocytic Leukaemia (APML)?

A

Rare subtype of AML characterised by over proliferation of promyelocytes

36
Q

Why is APML a haematological emergency?

A

Causes coagulopathy which can lead to CNS and pulmonary haemorrhaging

37
Q

Why does APML cause coagulopathy in 2 ways?

A

APML blasts express tissue factor and secrete IL-1, which activate clotting cascade

Cancer Procoagulant in present in APML cells and activates factor 10 directly

38
Q

Give 5 risk factors of APML?

A

Age: Risk increases as age increases (more common in middle age than children)

Hispanic

Obesity

Occupational exposure eg. Chemical agents

Previous chemotherapy or radiotherapy

39
Q

Give common signs and symptoms of APML?

A

Petechiae

Excessive mucocutaneous bleeding

Frequent infections and fever

Fatigue

Weight loss and appetite loss

Pain in joints and bones

Pallor

40
Q

Which 5 lab tests should be done to investigate APML?

A

FBC: Pancytopenia

Coagulation screen: High APTT and high PT

D-dimer test: High

Blood film

FISH genetic testing

41
Q

What is the characteristic finding of APML on a blood film?

A

Large, bi-lobed, hypergranular cells (buttock cells) with multiple auer rods

42
Q

When APML is suspected, what genetic testing must be done for rapid diagnosis?

A

FISH: PML-RARA fusion gene

Translocation between chromosomes 15 and 17 produces PML-RARA fusion gene

RARA codes for retinoid acid receptor alpha, which normally allows promyelocytes to differentiate: Fusion gene prevents this

43
Q

What is the emergency management of APML?

A

All-trans retinoic acid (ATRA)

Arsenic trioxide

44
Q

DIC

A

Clinical skills Yr2 (18 decks)

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